Use of the mechanical in-exsufflator in pediatric patients with neuromuscular disease and impaired cough

Chest. 2004 Apr;125(4):1406-12. doi: 10.1378/chest.125.4.1406.


Background: Impaired cough secondary to weakness from neuromuscular disease (NMD) can cause serious respiratory complications, including atelectasis, pneumonia, small airway obstruction, and acidosis. The mechanical in-exsufflator (MI-E) delivers a positive-pressure insufflation followed by an expulsive exsufflation, thereby simulating a normal cough. Use of the MI-E in adults with impaired cough results in improved cough flows and enhanced airway clearance. However, only limited reports of MI-E use in children exist.

Objective: To determine the safety, tolerance, and effectiveness of the MI-E in a pediatric population.

Method: Retrospective medical record review.

Participants: Sixty-two patients (34 male patients) observed in a pediatric pulmonary program with NMD and impaired cough in whom MI-E therapy was initiated. Median age at initiation of MI-E use was 11.3 years (range, 3 months to 28.6 years). Diagnoses included the following: Duchenne muscular dystrophy (17 patients); spinal muscular atrophy, types I and II (21 patients); myopathy (12 patients); other nonspecific NMD (12 patients). Mechanical ventilation via tracheostomy was used in 29 patients, and 25 patients used noninvasive ventilation.

Results: The median duration of use was 13.4 months (range, 0.5 to 45.5 months). One infant died before using MI-E at home. Five patients chose not to continue MI-E therapy. Complications were reported in two patients, but ultimately they used the MI-E device. Chronic atelectasis resolved in four patients after beginning MI-E therapy, and five patients experienced a reduction in the frequency of pneumonias.

Conclusion: In 90% of our study population, the use of an MI-E was safe, well-tolerated, and effective in preventing pulmonary complications.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Cough / physiopathology
  • Cough / therapy*
  • Exhalation
  • Female
  • Humans
  • Infant
  • Insufflation / instrumentation*
  • Male
  • Muscular Atrophy, Spinal / therapy
  • Muscular Dystrophy, Duchenne / therapy
  • Neuromuscular Diseases / therapy*
  • Pneumonia / prevention & control
  • Positive-Pressure Respiration / instrumentation*
  • Retrospective Studies
  • Safety